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Review
. 2021 Nov 25;23(1):288.
doi: 10.1186/s13075-021-02678-6.

The role of TGF-β or BMPR2 signaling pathway-related miRNA in pulmonary arterial hypertension and systemic sclerosis

Bei Xu  1 , Guanhua Xu  1 , Ye Yu  1 , Jin Lin  2
Affiliations
Review

The role of TGF-β or BMPR2 signaling pathway-related miRNA in pulmonary arterial hypertension and systemic sclerosis

Bei Xu et al. Arthritis Res Ther. .

Abstract

Pulmonary arterial hypertension (PAH) is a severe complication of connective tissue disease (CTD), causing death in systemic sclerosis (SSc). The past decade has yielded many scientific insights into microRNA (miRNAs) in PAH and SSc. This growth of knowledge has well-illustrated the complexity of microRNA (miRNA)-based regulation of gene expression in PAH. However, few miRNA-related SSc-PAH were elucidated. This review firstly discusses the role of transforming growth factor-beta (TGF-β) signaling and bone morphogenetic protein receptor type II (BMPR2) in PAH and SSc. Secondly, the miRNAs relating to TGF-β and BMPR2 signaling pathways in PAH and SSc or merely PAH were subsequently summarized. Finally, future studies might develop early diagnostic biomarkers and target-oriented therapeutic strategies for SSc-PAH and PAH treatment.

Keywords: Pulmonary arterial hypertension; Systemic sclerosis; miRNAs.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Flowchart of searching candidate miRNAs and related articles included in this study
Fig. 2
Fig. 2
The role of TGF and BMPR2 signaling in PAH. Abbreviation: TGF, transforming growth factor; BMPR2, Bone Morphogenetic Protein Receptor Type II; PAH, pulmonary arterial hypertension
Fig. 3
Fig. 3
The SSc-related miRNAs in TGF-β signaling and BMPR2 signaling. Abbreviation: TGF-β, Transforming growth factor-beta; BMPR2, SSc, systemic sclerosis; SMAD1/5/8, Mothers against decapentaplegic homolog 1/5/8; SMAD4 Mothers against decapentaplegic homolog 4
See this image and copyright information in PMC

References

    1. Orlandi M, Lepri G, Damiani A, Barsotti S, Di Battista M, Codullo V, et al. One year in review 2020: systemic sclerosis. Clin Exp Rheumatol. 2020;38(Suppl 125(3)):3–17. - PubMed
    1. Campo A, Mathai SC, Le Pavec J, Zaiman AL, Hummers LK, Boyce D, et al. Hemodynamic predictors of survival in scleroderma-related pulmonary arterial hypertension. Am J Respir Crit Care Med. 2010;182:252–260. - PMC - PubMed
    1. Chung L, Farber HW, Benza R, Miller DP, Parsons L, Hassoun PM, et al. Unique predictors of mortality in patients with pulmonary arterial hypertension associated with systemic sclerosis in the REVEAL registry. Chest. 2014;146:1494–1504. - PMC - PubMed
    1. Condliffe R, Kiely DG, Peacock AJ, Corris PA, Gibbs JS, Vrapi F, et al. Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med. 2009;179:151–157. - PubMed
    1. Kawut SM, Taichman DB, Archer-Chicko CL, Palevsky HI, Kimmel SE. Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis. Chest. 2003;123:344–350. - PubMed

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